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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 60  |  Issue : 1  |  Page : 74-77

Clinico – Epidemiological profile of uncommon manifestations of rhino orbital cerebral mucormycosis


Department of Ophthalmology, Tirunelveli Medical College, Palayamkottai, Tirunelveli, Tamil Nadu, India

Date of Submission01-Oct-2021
Date of Decision02-Nov-2021
Date of Acceptance06-Dec-2021
Date of Web Publication22-Mar-2022

Correspondence Address:
Dr. M Rita Hepsi Rani
Assistant Professor, Orbit Clinic, Department of Ophthalmology, Tirunelveli Medical College, Palayamkottai, Tirunelveli, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_151_21

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  Abstract 


Following the COVID pandemic, mucormycosis epidemic began as the next threat. A huge number of cases started accumulating. Although there is known pathogenesis and treatment for mucormycosis, several cases presented in uncommon ways causing a delay and dilemma in diagnosis and management. This case series is to highlight some of such presentations. Here, we present a series of nine cases which has deviated from the normal clinical progression of the sinus to the orbit. This case series depicts patients who had late signs of orbital cellulitis such as central retinal artery occlusion, papilledema, and optic neuritis as initial presentations. Furthermore, cases with superadded bacterial infection and aspergilloma are highlighted.

Keywords: Orbital cellulitis, papilledema, rhino-orbital mucormycosis


How to cite this article:
Hepsi Rani M R, Deepa M, Mathivadhani L S, Gopinayik P, Saradha M, Vedhavalli T, Sowmiya B, Akalya R, Uma M. Clinico – Epidemiological profile of uncommon manifestations of rhino orbital cerebral mucormycosis. TNOA J Ophthalmic Sci Res 2022;60:74-7

How to cite this URL:
Hepsi Rani M R, Deepa M, Mathivadhani L S, Gopinayik P, Saradha M, Vedhavalli T, Sowmiya B, Akalya R, Uma M. Clinico – Epidemiological profile of uncommon manifestations of rhino orbital cerebral mucormycosis. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Jul 2];60:74-7. Available from: https://www.tnoajosr.com/text.asp?2022/60/1/74/340355




  Introduction Top


Mucormycosis is caused by the fungi of Mucorales family. Various manifestations of mucormycosis have been documented in the order of decreasing incidence as (1) rhino-orbital, (2) cerebral, (3) pulmonary, (4) cutaneous, (5) gastrointestinal, and (6) disseminated.[1]

Fungal hyphae exhibit the property of angio-invasion by utilizing the iron from heme molecules and cause mycotic thrombus, which leads to ischemic necrosis.[2] This prevents the migration of neutrophils and antifungals and thereby favoring fungal growth. Furthermore, retrograde extension along nerves and perineural spread occurs.

Upon inhalation of fungal spores, there occurs its germination. The fungal hyphae extend rapidly into adjacent tissues in predisposed individuals. The locoregional spread of fungal infection is elucidated in [Figure 1].[3]
Figure 1: Locoregional spread of fungal infection

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Patients initially present with signs of sinusitis such as headache, nasal discharge, epistaxis, and blackish discoloration of the nasal mucosa.[4] On extension to orbital structures, they present with periorbital pain, edema, ptosis, proptosis, internal or external ophthalmoplegia, and loss of vision. On inferior spread from the maxillary sinus floor there can occur loosening of teeth, palatal swelling, blackish discoloration over the palate.

This study is case series depicting the varied presentations of mucormycosis deviating the usual route.


  Case Reports Top


Case 1: A white-eyed orbital cellulitis

A 55-year-old, COVID-recovered, known diabetic presented with sudden-onset pain, loss of vision OD for 1 day. On examination, the vision was Perception of light +, with relative afferent pupillary defect (RAPD) in the right eye. Rest of the examination was normal with a white eye, as shown in [Figure 2]a. A suspicion of retrobulbar optic neuritis arose, but owing to the presence of predisposing factors of Mucor, she was subjected to computed tomography (CT) paranasal sinuses (PNS) and orbit, which showed right posterior ethmoid and sphenoid sinus mucosal thickening. There was no evidence of bony destruction. The patient was started on insulin and antibiotic prophylaxis. Two days later, she worsened and developed lid edema, external ophthalmoplegia, and no PL and pansinusitis on the right side, as depicted in [Figure 2]b.
Figure 2: (a) Day 1 shows white eye. (b) Day 3 shows lid edema ptosis

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Case 2: Mucor with superadded antibioma

A 72-year-old female post-COVID, post mucormycosis, was treated with functional endoscopic sinus surgery (FESS) for bilateral ethmoid sinusitis with amphotericin and ceftriaxone. Later, she developed a localized swelling in both upper eyelids for 5 days, as depicted in [Figure 3]a. There were no other signs of orbital cellulitis such as ptosis, congestion, chemosis, or ophthalmoplegia. CT revealed mild mucosal thickening in the bilateral ethmoid and maxillary sinus with loculated soft-tissue swelling of the upper eyelid. Excision biopsy was performed [Figure 3]b. Histopathological analysis revealed antibioma. Hence, this is a case with superadded bacterial infection which has responded to antibiotics and becomes a localized swelling as antibioma.
Figure 3: (a) Localized swelling of both eye upper lids. (b) Resolution of swelling postantibiotic treatment

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Case 3: Mucormycosis presenting as central retinal artery occlusion

A 50-year-old woman, a case of Mucor involving ethmoid sinus treated with FESS, 2 weeks later presented with sudden loss of vision in Right eye (RE) for 2 days. O/E, orbits appeared normal, vision OD was PL+, and fundus showed RE central retinal artery occlusion (CRAO), as shown in [Figure 4]a. CRAO is commonly observed as end stage in orbital apex syndrome. However, this patient presented with CRAO as an initial manifestation of orbital involvement. The cause in this case is suspected as angio-invasion of the central retinal artery from the growth in the ethmoid sinus.[5]
Figure 4: Fundus picture depicting central retinal artery occlusion

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Case 4: Mucormycosis presenting as papilledema

The patient, depicted in [Figure 5]a, is a post-COVID, right maxillary fungal sinusitis post inferior maxillectomy, treated with IV L-amphotericin B, followed by tablet posaconazole oral maintenance. On follow-up, the patient came with complaints of headache. External examination appeared normal with no orbit findings. Clinical examination was normal except for chronic papilledema, as shown in [Figure 5]b and [Figure 5]c. The patient during the initial days had a history of loosening of teeth, so the probable cause for chronic papilledema could be dental spread to the anterior cranial fossa.[6]
Figure 5: (a) Clinical picture showing no orbital involvement. (b and c) Chronic papilledema

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Case 5: Mucormycosis presenting as a chalazion

A 40-year-old diabetic, post-COVID, post-FESS for right maxillary and ethmoid sinusitis presented 10 days later with a nonerythematous, localized swelling in the lower lid, as shown in [Figure 6]a. Repeat CT PNS showed residual maxillary sinusitis. Hence, repeat FESS was done. Subsequently, the swelling reduced on its own [Figure 6]b. This swelling is suspected to be a granuloma which resolved with antifungals.
Figure 6: (a) Clinical picture showing a soft cystic swelling in the lower lid. (b) Postoperative clinical picture of the patient following functional endoscopic sinus surgery with disappearance of the cystic swelling

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Case 6: Cutaneous mucormycosis

A 62-year-old woman, post-COVID, diabetic presented with right maxillary and ethmoid fungal sinusitis and a blackish eschar over the right lower lid with induration, as shown in [Figure 7]a. Examination revealed normal vision with no features of orbital cellulitis such as chemosis and ophthalmoplegia. CT orbit showed mild preseptal thickening. The case was initially suspected as orbital cellulitis with overlying eschar, but the presence of normal orbital finding clinically and radiological created a need for further evaluation. Hence, a skin biopsy was taken, which showed necrotizing granulomatous tissue with fungal hyphae making the diagnosis as cutaneous mucormycosis.[7] The patient was done an excision biopsy of eschar. The postoperative outcome is shown in [Figure 7]b.
Figure 7: (a) Eschar over the right eyelid with surrounding induration. The eye appears white with to congestion or chemosis. (b) The postoperative picture following excision biopsy

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Case 7: Mucormycosis with pulmonary aspergilloma

A 55-year-old male presented with preseptal cellulitis, and later, he developed cough and hemoptysis for 2 days. He was admitted to the COVID suspect ward, and CT was taken for COVID status. CT chest showed bilateral lower lobe consolidation with fungal ball in the left lower lobe, as shown in [Figure 8]. Owing to the Mucor pandemic and clinical suspicion, bronchoscopy was done. Bronchoscopic lavage showed fungal hyphae in the right lung aspirate. The patient was started on L-amphotericin B. Now, the patient is monitored on biweekly bronchoscopy and maintenance tablet posaconazole.[8]
Figure 8: Computed tomography chest shows left lower lobe fungal ball (arrow)

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  Discussion Top


The first case of white-eyed orbital cellulitis highlights the significance of close anatomical proximity of the sphenoid sinus to the orbital canal. With no other signs of orbital cellulitis, the patient presented only with RAPD at the initial stage.

The second case of antibioma was suspected as a recurrence of Mucor initially. However, a timely clinical suspicion and the deployment of histopathological analysis have avoided the need of extra drugs and its adverse effects.

The third case scenario of mucormycosis presenting as CRAO initially depicts yet another proof of angio-invasive nature of mucormycosis.

The fourth case with papilledema shows us that even with minimal infective load, cerebral spread could occur through a dental route.

The fifth case scenario shows us the residual infection instead of producing diffuse reaction and widespread could be concealed by our immune system into a localized granulomatous reaction.

In the sixth case, cutaneous mucormycosis has occurred following inoculation of fungal spores over the abraded skin. In the presence of predisposing factors, the spores have germinated and become an eschar.

The seventh case of pulmonary mucormycosis shows us that the deviated spread of mucormycosis to brain not following the rhino-orbital-cerebral route.


  Conclusion Top


The outbreak of Mucor endemic in India was unexpected and unprepared. A large number of cases posed a burden to us. Just like a mutated virus, Mucor also presented in different dimensions, some being uncommon, as we have discussed. However, a good clinical anticipation owing to the multitude of cases and the combined efforts of multidisciplinary medical branches has helped us curb the endemic.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dubey S, Mukherjee D, Sarkar P, Mukhopadhyay P, Barman D, Bandopadhyay M, et al. COVID-19 associated rhino-orbital-cerebral mucormycosis: An observational study from Eastern India, with special emphasis on neurological spectrum. Diabetes Metab Syndr 2021;15:102267.  Back to cited text no. 1
    
2.
Ibrahim AS, Spellberg B, Walsh TJ, Kontoyiannis DP. Pathogenesis of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S16-22.  Back to cited text no. 2
    
3.
Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S23-34.  Back to cited text no. 3
    
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Mitra S, Janweja M, Sengupta A. Post-COVID-19 rhino-orbito-cerebral mucormycosis: a new addition to challenges in pandemic control. Eur Arch Otorhinolaryngol. 2021 Jul 26:1–6. doi: 10.1007/s00405-021-07010-1. Epub ahead of print. PMID: 34309754; PMCID: PMC8312209.  Back to cited text no. 4
    
5.
Bawankar P, Lahane S, Pathak P, Gonde P, Singh A. Central retinal artery occlusion as the presenting manifestation of invasive rhino-orbital-cerebral mucormycosis. Taiwan J Ophthalmol 2020;10:62-5.  Back to cited text no. 5
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6.
Jha R, Gude D, Chennamsetty S, Kotari H. Intracranial hypertension: An unusual presentation of mucormycosis in a kidney transplant recipient. Indian J Nephrol 2013;23:130-2.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Arnáiz-García ME, Alonso-Peña D, González-Vela Mdel C, García-Palomo JD, Sanz-Giménez-Rico JR, Arnáiz-García AM. Cutaneous mucormycosis: Report of five cases and review of the literature. J Plast Reconstr Aesthet Surg 2009;62:e434-41.  Back to cited text no. 7
    
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Agrawal R, Yeldandi A, Savas H, Parekh ND, Lombardi PJ, Hart EM. Pulmonary mucormycosis: Risk factors, radiologic findings, and pathologic correlation. Radiographics 2020;40:656-66.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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